tbuckley/cabot-search huggingface dataset:
P-085: THE MORBIDITY AND MORTALITY OF SICKLE CELL DISEASE PATIENTS IN THE HAEMATOLOGY DEPARTMENT OF THE IGNACE DEEN HOSPITAL, CONAKRY HOSPITAL AND UNIVERSITY CENTRE
Authors: DIAKITE M., GUILAVOGUI F., KANTE A., CONDE A., SYLLA M., TOUNKARA T.
Journal: HemaSphere (2022)

Purpose: Objective: To describe the morbidity and mortality of sickle cell disease patients in the Hematology Department of the Ignace Deen National Hospital in Conakry. Materials and methods: This was a retrospective descriptive and analytical study that took place over a period of 7 years from January 1, 2013 to December 31, 2019. The study population consisted of records of sickle cell patients hospitalized during the study period. Results: 150 records of hospitalized sickle cell patients were selected with 67.33% male and a sex ratio of 2.06. Their ages ranged from 15 to 65 years, with a mean of 29.17 years. The age group under 25 years represented 45.33%. Students accounted for 42%. The main reasons for hospitalization were anemic syndrome (93.33%), osteoarticular pain (67.33%), infectious syndrome (91.33%), hemolytic crises (32.67%) and acute chest syndrome (28.66%). Sickle cell disease-related mortality was 5.33%. The major causes of death in hospitalized sickle cell patients were severe anemia (100.0%) and infection (50.0%). Conclusion: It emerges from our study that the morbi-mortality of sickle cell disease is non-negligible, marked by acute and chronic complications including severe anemia and infections. Early diagnosis and regular monitoring of patients with adequate care would minimize them. The authors do not declare any conflict of interest
P-074: NUTRITIONAL AND PUBERTAL PROFILE OF CHILDREN WITH MAJOR SICKLE CELL DISEASE ADMITTED AT THE MEDICAL AND HEALTH COUNCIL CENTER OF CONAKRY, GUINEA
Authors: DIALLO M., CHERIF M., DOUKOURE M., DIALLO F., BALDE N.
Journal: HemaSphere (2022)

Purpose: A child’s growth is a reflection of it nutritional and pubertal status and its proven that nutrition is a factor affecting pubertal development. Sickle cell patients often have slowed growth and delayed pubertal development. This work aims to assess the nutritional and/or pubertal profile in children with sickle cell diseases (SS) admitted at the CEMECO center. Materials and methods: This was a cross-sectional study and study participant under 16 years were randomly selected from the health center database having about 6497 cases and enrolled in the study.The participants were divides into two groups based on the electrophoresis of hemoglobin: Sickle cell disease including 103 cases of SS (homozygote) and 18 cases of SC, S, β-Thalassemia and SE (heterozygous). While the group of non-sickle cell participants includes 87 (AA and AS). Results: We included 208 children among them121 sickle cell disease patients and 87 non sickle cell diseasechildren. with a sex ratio M/F was about1.02. The meanage of sickle cell patients was 8.7±4.4 years while that of non-sickle cell patients was 9.5 ± years. The family income evaluated according to the MICS, was similar between the two groups (P= 0.123). Evaluation of nutritional status revealed that in children under five years of age, the weight / height index z-score of sickle cell patients (-0.82 ± 1.1 [-3.01-1.62]) was lower than that of non-sickle cell patients (-0.29 ± 1.4Zscore {-2.91-2.86]). However, the difference was not significative (P = 0.155).The height/age ratio express as Z score showed a significative difference between sickle cell patients (1.1 Z score)while non-sickle cell patients (-0.2 Z score) (P =0.000). Underweight evaluated by weight/age z-score showed that the in-sickle cell patients were -0.91 and -0.12 in non-sickle cell patients (P=0.014). However, the BMI evaluation did not show any significant difference between sickle and non-sickle cell patients P=0.188. The proportion of delayed puberty were 4 (4.8%) in sickle cell patients compared to 6.3% in non-in sickle cell patients. puberty. Uni-variate analysis showed an association between sickle cell disease and testicular development (P=0.046). Conclusion: The results of our study highlighted growth retardation and slowed testicular development in children with sickle cell disease. The authors do not declare any conflict of interest
P-050: UNDERSTANDING BARRIERS TO AND ENABLERS IN EMPLOYMENT FOR PEOPLE WITH SICKLE CELL DISORDERS IN ENGLAND
Authors: BERGHS M., DYSON S.
Journal: HemaSphere (2022)

Purpose: This paper reports on empirical research which was conducted in England in 2018-2020 before the COVID-19 pandemic. It involved trying to understand barriers and enablers to employment for people who have sickle cell disorder (SCD). Materials and methods: The project worked with people with SCD, and two of their voluntary organisations, the Sickle Cell Society and OSCAR Sandwell as partners. It used a method of ‘democratic co-production’ and people with SCD led two focus group discussions to formulate and critique questions to ask about work and to share their understandings of what barriers and enablers to employment had been in their experiences. Then forty-seven individual interviews were conducted with people with SCD incorporating feedback from those focus groups Results: We found that work was about more than just employment for people with SCD who had to engage in self-management of their conditions and were contributing a lot to their families and communities. There were considerable barriers to access employment and once in employment people could face various forms of discriminations. However, reasonable adjustments for peope with SCD in the workplace were relatively simple and inexpensive to put into practice. While people with SCD have a right to accomodations in the workplace they did not often understand legislation or how that affected things like disclosure or their rights. Conclusion: We development an employment policy guide to aid employers and employees in the workplace in England. Post-COVID-19 we have also thought about how to apply this to flexible work and working from home. The authors do not declare any conflict of interest
P-096: ACUTE SICKLE CELL INTRAHEPATIC CHOLESTASIS: RARE DISEASE WITH UNUSUAL PRESENTATION, LESSONS LEARNT FROM AN INTERESTING CASE.
Authors: SIHARE P.
Journal: HemaSphere (2022)

Purpose: Management of Sickle cell disease in India the is evolving. The pediatric fatal disease is shifting to adult chronic organ disease. Sickle hepatopathy is an umbrella term which covers hepatic sickle crisis, acute hepatic sequestration crisis although uncommon but manageable. Acute sickle cell intrahepatic cholestasis (SCIC) is a rare phenomenon, difficult to treat and has very high mortality. The pathogenesis of Acute sickle cell intrahepatic cholestasis involves sickling within the hepatic sinusoids leading to vascular stasis, hypoxia, and ballooning of the hepatocytes, resulting in intracanalicular cholestasis. We describe a difficult case of Acute sickle cell intrahepatic cholestasis which has a subacute course, challenges faced, and lessons learnt from managing it. Materials and methods: A 14-year-old girl with sickle cell anemia who was non-compliant with hydroxyurea treatment presented to us with complaints of pain in the right side of abdomen, drowsiness, lethargy, nausea and loss of appetite. She was transfused twice at another center before reaching our center. On examination, she was disoriented, febrile, icteric, severely pale with active bleeding from gums and mouth and moderate hepatosplenomegaly. Hemoglobin was 7.2 grams/ dl with reticulocyte count of 2%, with neutrophilic leukocytosis and normal platelet count. Hemoglobin S was 42%. Serum bilirubin was 68 mg/dl with direct fraction of 65.3mg/dl. SGPT and SGOT were 263 and 202 IU/dl respectively and prothrombin time was raised with INR of 1.79. Viral hepatitis A, B, C and E were ruled out. Bacterial infections were ruled out. The possibility of Acute sickle cell intrahepatic cholestasis was considered, and the child was managed with partial exchange transfusion till Hemoglobin S was brought to <10%. She also received fresh frozen plasma in view of raised prothrombin time and active bleeding. Results: She did not improve until 14 days after admission.Gradually after 20 days she started talking and eating. However, liver enzymes and bilirubin normalized only after 40 days. She was discharged in stable condition and is continued on follow up. Conclusion: Sickle cell hepatopathy is an umbrella diagnosis for all the varied causes of hepatic dysfunction ranging from sickling crisis to transfusion transmitted viral infections. In our case, viral hepatitis was ruled out conclusively.A very rare condition Acute sickle cell intrahepatic cholestasis was considered in view of the clinical presentation serum bilirubin was extremely high and mildly elevated transaminases. However. Hepatic sequestration was unlikely based on the hemoglobin level and reticulocyte count. Differentiating the varied causes in resource constrained centers can be often a daunting task and multipronged management needs to be initiated at the earliest to ensure a positive outcome. The authors do not declare any conflict of interest
P-056: SICKLE CELL DISEASE: DIAGNOTIC CHALLENGES AND FUTURE PERSPECTIVE IN NICARAGUA
Authors: PERNUDY UBAU A., CRUZ ALEMÁN S., SALINAS MOLINA J., REQUENEZ Y.
Journal: HemaSphere (2022)

Purpose: Nicaragua is a developing country located in Central America. The country has a multiethnic population, with Mestizos, American Indian, and black being the most predominant ethnic groups. Generally, there is a lack of national screening programs as well as a research center dedicated to the study of hemoglobinopathies. Commonly, solubility is used as screening test. Other Diagnostic methods such as electrophoresis or HPLC are restricted to a very small number of patient due to their high cost and availability. Therefore, Sickle cell disease (SCD) is diagnosed on the basis of the patient’s clinical examination, history, and routine hematologic analysis. Altogether, the deficient facilities, expertise, and economical limitations contribute to the unknown prevalence of this pathology. Recently, great efforts have been made to carry out screening studies in different parts of the country. Overall more than 800 were tested by using cellulose acetate electrophoresis (CAE). The aims of this study are to estimate the prevalence of homozygotes and heterozygotes as well as analyze the accuracy of the clinical diagnostic. Additionally, to foreseen the SCD distribution statistical analysis will be performed Materials and methods: Hemoglobin lysate from peripheral blood was obtained and cellulose acetate electrophoresis was carried out to define the hemoglobin phenotype and to confirm the clinical diagnostic. Descriptive statistics and probabilistic graphical model (Markov influence diagrams) were used to calculate the SCD prevalence and to foresee SCD distribution, respectively. Results: In the first group, 150 blood samples from patients with a clinical diagnosis of SCD were analyzed over a 2-year-long study period. Sixty-five patients (43%) with the hemoglobin phenotype of SCD were confirmed. However, 85 patients (57%) had an SCT or normal phenotype. In the second group, 450 blood samples from healthy individuals were screened, 21 (4.6%) were positive for SCT, and the other 429 (95.4%) were normal Conclusion: Although significant advances have been made in the diagnostic and prevention of SCD, in low-income countries such as Nicaragua, there is a great need for resources including newborn screening and counseling programs, specialized and equipped laboratories, and training for healthcare professionals. In this context, epidemiological studies, for instance, the one here presented are vital to assess the current status of the disease and can provide guidance for future clinical care of the patient with SCD The authors do not declare any conflict of interest
P-080: IMPLEMENTATION OF SCREENING TOOL IN SICKLE CELL CLINIC TO IDENTIFY SOCIAL DETERMINANTS OF HEALTH
Authors: PATEL J., MANDAVA M., NOISETTE L., DIAZ C., BORAD A., JACKSON S., BERGMANN S.
Journal: HemaSphere (2022)

Purpose: Social determinants of health (SDOHs) are the conditions in which people are born into and surrounded by throughout their life. Generally, these include economic stability, education, social and community context, health and health care and housing/ neighborhood characteristics. In the pediatric population, it is these factors that are thought to influence health, well being and development. Additionally, patients with sickle cell disease face significant complications from their chronic disease and racial disparities, which may contribute to effects of adverse SDOH. Prior studies have suggested the importance of implementing a universal screening to identify adverse SDOHs in order to provide necessary resources. Materials and methods: In this prospective, quality improvement initiative, we administered a universal screener for social determinants of health in our pediatric sickle cell clinic. The paper questionnaire included screening about housing and food insecurity, transportation difficulties and education concerns. The aim of the study was to determine feasibility of administering screening in pediatric sickle cell clinic and to better understand the socio-economic needs of our sickle cell disease population. Results: Data was collected between August 2020 to May 2021, during which time 226 screening questionnaires were completed. With each PDSA cycle, percentage of patients screened increased over time and by May 2021, we achieved about a 35% screening rate. When we looked at specific needs, housing insecurities and transportation difficulties were reported the most, at 39.6% and 44.4% respectively. This data allowed us to identify and provide additional resources for patients. Conclusion: The clinical complications of sickle cell disease place extensive burdens on patients. These strains are additionally exacerbated by significant adverse SDOHs that these patients often face. It is feasible to implement a universal screening tool in a pediatric sickle cell clinic, to identify these adverse events. This information can be used to provide appropriate resources and follow up for these patients. We hope to look at this data in more detail to evaluate the success of providing additional resources.Monthly run chart to compare percentage of patients surveyed and percentage of patients previously surveyed in the month The authors do not declare any conflict of interest
Clinician communication strategies associated with increased uptake of the human papillomavirus (<scp>HPV</scp>) vaccine: A systematic review
Authors: Catherine Constable, Kyle Ferguson, Joey Nicholson, Gwendolyn P. Quinn
Journal: CA A Cancer Journal for Clinicians (2022)

Abstract Human papillomavirus (HPV) is currently linked to almost 35,000 new cases of cancer in women and men each year in the United States. Gardasil‐9 (Merck &amp; Company), the only HPV vaccine now available in the United States, is nearly 100% effective at preventing precancers caused by oncogenic HPV types. In the United States, however, only about one half of adolescents are up to date with HPV vaccination. It is well known that health care clinicians’ recommendations play a significant role in parents’ decisions regarding HPV vaccination. A growing body of literature examines specific communication strategies for promoting uptake of the HPV vaccine. A comprehensive review of the evidence for each of these strategies is needed. The authors searched the PubMed, EMBASE, Cochrane Central Register of Controlled Trials, PsycINFO, Cumulative Index to Nursing and Allied Health Literature, and Web of Science Complete databases for original articles with a defined clinician communication strategy and an outcome of HPV vaccine uptake or intention to vaccinate (PROSPERO registry no. CRD42020107602). In total, 46 studies were included. The authors identified two main strategies with strong evidence supporting their positive impact on vaccine uptake: strong recommendation and presumptive recommendation . Determinations about a causal relationship were limited by the small numbers of randomized controlled trials. There is also opportunity for more research to determine the effects of motivational interviewing and cancer‐prevention messaging.
Tobacco industry ownership of pharmaceutical companies: an international survey of people with respiratory disease
Authors: Ruth Tal-Singer, Linda Walsh, Tim Deuby, Angela Shaughnessy, Karin F Hoth, Victor Gascon, Susanne Hintringer, Nicole Hass, Tonya Winders, Lucy Morgan, Byron Thomashow, David M Mannino
Journal: Thorax (2022)

The 2021 purchase of the respiratory pharmaceutical company Vectura by Phillip Morris International has been criticised by the public health and medical community, as a conflict of interest, with little input to date, from the patient community or the public. To address this gap, the COPD Foundation, along with global partners, surveyed 1196 people with chronic respiratory disease. 70% were bothered by a tobacco company making an inhaler to treat lung conditions and 48% reported that they would want to switch inhalers if they knew that a tobacco company made or sold their inhaler devices. Patients care about who makes the therapies used to treat their diseases.
Long‐term changes in milk component immunoglobulins reflect milk oral immunotherapy outcomes in Finnish children
Authors: Tiina Kaisa Kauppila, Victoria Hinkkanen, Terhi Savinko, Piia Karisola, Anna Kaarina Kukkonen, Marita Paassilta, Anna S. Pelkonen, Mika J. Mäkelä
Journal: Allergy (2022)

Milk oral immunotherapy (OIT) may increase the amount of milk protein that can be ingested without triggering an allergic reaction. It is important to understand why some patients benefit from the treatment while others do not.The aim was to define the differences in the milk allergen component-specific (casein, α-lactalbumin, ß-lactoglobulin) immunoglobulin (sIg [sIgE, sIgG4, and sIgA]) levels relative to the long-term outcomes of milk OIT.In this long-term, open-label follow-up study, 286 children started milk OIT between 2005 and 2015. Follow-up data were collected at two points: the post-buildup phase and long term (range 1-11 years, median 6 years). Comparisons of sIg levels were made among three outcome groups of self-reported long-term milk consumption (high-milk dose, low-milk dose, and avoidance).A total of 168 (59%) of the 286 patients on OIT participated. Most patients (57%) were in the high-dose group; here, 80% of these patients had a baseline casein sIgE value less than 28 kUA/L, they had the lowest casein sIgE levels at all time (p < .001), their casein sIgG4/IgE levels increased, and long-term casein sIgA was highest compared with the low-dose and avoidance groups (p = .02). Low-milk dose group had the highest casein sIgG4/IgE levels in long term (p = .002).The baseline Ig profiles and responses to milk OIT differed depending on long-term milk consumption. Lower casein sIgE levels were associated with better outcome. Milk casein sIgA differed in the long term among high-milk consumers.
Syndrome of Combined Pulmonary Fibrosis and Emphysema: An Official ATS/ERS/JRS/ALAT Research Statement
Authors: Vincent Cottin, Moises Selman, Yoshikazu Inoue, Alyson W. Wong, Tamera J. Corte, Kevin R. Flaherty, MeiLan K. Han, Joseph Jacob, Kerri A. Johannson, Masanori Kitaichi, Joyce S. Lee, Alvar Agusti, Katerina M. Antoniou, Pauline Bianchi, Fabian Caro, Matias Florenzano, Liam Galvin, Tae Iwasawa, Fernando J. Martinez, Rebecca L. Morgan, Jeffrey L. Myers, Andrew G. Nicholson, Mariaelena Occhipinti, Venerino Poletti, Margaret L. Salisbury, Don D. Sin, Nicola Sverzellati, Thomy Tonia, Claudia Valenzuela, Christopher J. Ryerson, Athol U. Wells
Journal: American Journal of Respiratory and Critical Care Medicine (2022)

Section:ChooseTop of pageAbstract <<IntroductionMethodsHistorical PerspectiveEpidemiologyEtiologiesClinical Manifestations a...Lung FunctionImaging FeaturesPathology FeaturesOutcome and ComplicationsPathogenesis and Putative...Terminology and Definitio...Is CPFE a Syndrome?ManagementClinical Trial Perspectiv...Relevance of CPFE for the...Research PrioritiesConclusionsReferencesCITING ARTICLES
Cortical Hemiballismus Associated With an Insular and Temporal Lobe Infarct
Authors: Cleo Zarina Reyes, Ramiro Castro-Apolo, Yevgeniy Isayev
Journal: JAMA Neurology (2022)

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Study Quality Must Be Considered When Evaluating Long-term Outcomes of Antenatal Corticosteroid Therapy in Children
Authors: Laura Schummers, Jennifer A. Hutcheon
Journal: JAMA Pediatrics (2022)

Our website uses cookies to enhance your experience. By continuing to use our site, or clicking "Continue," you are agreeing to our Cookie Policy | Continue JAMA Pediatrics HomeNew OnlineCurrent IssueFor Authors Podcast Publications JAMA JAMA Network Open JAMA Cardiology JAMA Dermatology JAMA Health Forum JAMA Internal Medicine JAMA Neurology JAMA Oncology JAMA Ophthalmology JAMA Otolaryngology–Head & Neck Surgery JAMA Pediatrics JAMA Psychiatry JAMA Surgery Archives of Neurology & Psychiatry (1919-1959) JN Learning / CMESubscribeJobsInstitutions / LibrariansReprints & Permissions Terms of Use | Privacy Policy | Accessibility Statement 2023 American Medical Association. All Rights Reserved Search All JAMA JAMA Network Open JAMA Cardiology JAMA Dermatology JAMA Forum Archive JAMA Health Forum JAMA Internal Medicine JAMA Neurology JAMA Oncology JAMA Ophthalmology JAMA Otolaryngology–Head & Neck Surgery JAMA Pediatrics JAMA Psychiatry JAMA Surgery Archives of Neurology & Psychiatry Input Search Term Sign In Individual Sign In Sign inCreate an Account Access through your institution Sign In Purchase Options: Buy this article Rent this article Subscribe to the JAMA Pediatrics journal